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Biomedical subjects

Celia Chen

Publications and source records attributed to Celia Chen.

13 recordsLinked to original sources

Toenail mercury and dietary fish consumption.

New England is one of three areas in the United States with the highest annual deposition of mercury, an established environmental pollutant with a variety of health effects. We measured the mercury content in toenails of 27 individuals in New Hampshire who participated as controls in a health study in 1994-95. The mean total toenail mercury concentration was 0.27 mcg/g (median 0.16; SD 0.27; range 0.04-1.15 mcg/g). The best predictor of toenail mercury levels was the mean combined fish and shellfish consumption measured using four simple questions from a validated food frequency questionnaire. Toenail total mercury content was significantly correlated with the mean average weekly consumption of finfish and shellfish (Spearman correlation coefficient 0.48, P=0.012). Multivariate models confirmed that toenail total mercury concentration was best predicted by total finfish and shellfish consumption.

Animals↗

Surgical margins for lentigo maligna and lentigo maligna melanoma: the technique of mapped serial excision.

OBJECTIVES: To assess the margins required for excision of lentigo maligna (LM) and lentigo maligna melanoma (LMM) by the technique of mapped serial excision (MSE), and to assess the efficacy of MSE. DESIGN: An interventional, prospective, noncontrolled case series. SETTING: Tertiary referral, dermatologic surgery unit. PATIENTS: Consecutive patients with head and neck LM or LMM who underwent MSE between March 1, 1993, and October 31, 2002. INTERVENTION: The MSE of LM or LMM. MAIN OUTCOME MEASURES: The number of 5-mm levels for excision of LM and LMM and recurrence. RESULTS: One hundred sixty-one LMs or LMMs in 155 patients were treated. Thirty percent (37 of 125) of LMs required more than 5-mm margins. For LMMs less than 1 mm in Breslow thickness, 12% (4/32) required more than 10-mm margins. For primary tumors, 20% of LMs (18 of 91) required more than 5-mm margins, while 10% of LMMs less than 1 mm in Breslow thickness (2 of 21) required more than a 10-mm margin. For recurrent tumors, 56% of LMs (19/34) required more than a 5-mm margin. Mean follow-up of 38 months (range, 5-100 months) showed 4 recurrences (2%) after MSE. The extrapolated recurrence at 5 years was 5.0%. CONCLUSIONS: The current recommendations of 5-mm margins for LM and 10-mm margins for LMM less than 1 mm in Breslow thickness are often insufficient. Our results demonstrate the importance of margin-controlled excision, particularly in recurrent lesions. The use of MSE offers a high cure rate, in conjunction with tissue conservation.

Adult↗

Late reactivation of thyroid orbitopathy.

BACKGROUND: Thyroid orbitopathy is characterized by spontaneous remissions and exacerbations. Although data on the natural history are scarce, the active phase is usually self-limited, encompassing an average period of 18-36 months. Late reactivation, defined as active orbitopathy occurring after more than 5 years of quiescent disease, appears to be an uncommon and poorly documented event. The aim of the present study was to assess the incidence and characteristics of late reactivation of thyroid orbitopathy within a tertiary referral orbital clinic. METHODS: A retrospective case review was conducted of patients with thyroid orbitopathy seen at the Royal Adelaide Hospital Orbital Clinic between 1996 and 2001. RESULTS: From 193 cases of thyroid orbitopathy, eight cases (5%) of late reactivation were identified. The mean age of patients was 51 years (range 32-76 years) with a female to male ratio of 3:1. All cases presented with proptosis and seven demonstrated increased diplopia and gaze restriction. Signs of soft tissue inflammation were seen in three cases. All were euthyroid at presentation with a past history of thyroid orbitopathy and hyperthyroidism. One patient had an association of smoking with reactivation but the remainder had no discernible precipitants. The average interval between the initial orbitopathy and reactivation was 12 years (range 6-30 years). Computed tomography revealed enlarged extraocular muscles in seven cases and two showed partial response to treatment with low dose radiotherapy and steroids. The average length of disease activity was 14 months (range 9-18 months). CONCLUSION: Late reactivation of thyroid orbitopathy appears to be an uncommon phenomenon. It may occur under euthyroid conditions with no obvious precipitants and often presents as a reactivation of myopathy.

Adult↗

Endoscopic approach to orbitofrontal cholesterol granuloma.

A 54-year-old man presented with an orbitofrontal cholesterol granuloma arising in the left frontal bone and abutting the dura. The lesion was successfully removed with an anterior orbitotomy via a superior lid crease incision. A 70-degree rigid endoscope was used to visualize removal of the granuloma from the inner surface of frontal bone and the dura. The patient made an uncomplicated recovery and there was no recurrence at two years follow-up. A combined anterior orbital and endoscopic approach may be an alternative to either frontal craniotomy or lateral orbitotomy in selected cases of orbitofrontal cholesterol granuloma.

Cholesterol↗

Endoscopic modified lothrop procedure: an alternative for frontal osteoma excision.

PURPOSE: To describe three cases of frontal osteoma which were safely removed with a stereotactic-assisted, endoscopic modified Lothrop approach (EMLP). METHOD: Retrospective case review. RESULTS: Three patients presented with extensive frontal sinus osteomas. Two underwent an EMLP and one patient with osteoma extending significantly into the orbit had an EMLP for removal of the medial frontal sinus component combined with an anterior orbitotomy to remove the orbital extension. In all cases stereotactic localization was utilized to ensure preservation of the skull base. Post-operatively, one patient required debridement of oedema at the junction of a lateral mucocele and the frontal sinus. The other two patients remained well post-operatively with no complications. At follow-up, there was no tumour recurrence. CONCLUSION: A stereotactic-assisted, EMLP with or without an orbital approach may be considered for the removal of large frontal osteomas and may provide a less invasive alternative to traditional external approaches.

Adult↗

Hereditary hyperferritinemia-cataract syndrome: prevalence, lens morphology, spectrum of mutations, and clinical presentations.

OBJECTIVES: To provide a comprehensive description of the clinical presentations, cataract morphology, and molecular basis of hereditary hyperferritinemia-cataract syndrome (HHCS) in 4 Australian pedigrees and to estimate its prevalence. METHODS: All known cases of HHCS in southeastern Australia were ascertained. Family members provided a medical history and underwent physical examination, lens photography, and venipuncture for measurement of serum ferritin levels and DNA extraction. Sequence analysis of the iron-responsive element of the ferritin light chain on chromosome 19q13.3-qter was performed. RESULTS: We investigated 26 affected individuals from 5 Australian pedigrees. Two pedigrees with HHCS ascertained independently were subsequently found to form 1 large kindred carrying the mutation A40G. The minimum estimated prevalence of HHCS is 1/200000. One pedigree had the mutation G32C. Among 2 smaller pedigrees studied, one carried a novel mutation (C39A), and the other was identified through the 2-year-old propositus with cataract but no positive family history. The latter case was shown to be due to a de novo mutation (G32U). All cataracts were highly distinctive in morphology, consisting of slowly progressive flecks, vacuoles, and distinctive crystalline deposits scattered predominantly in the lens cortex but also in the nucleus. Eight of 18 affected individuals examined have required cataract extraction to date. No other identified clinical manifestations of HHCS were delineated. CONCLUSIONS: Cataract morphology in HHCS is highly distinctive. Longitudinal observation demonstrated slow progression of the cataracts. This study highlights that, although HHCS is an autosomal dominant condition, the diagnosis should be considered even in sporadic cataract of typical morphology. Furthermore, individuals with unexplained hyperferritinemia should be referred for ophthalmological assessment, as the cataract may be asymptomatic but lead to a correct diagnosis of HHCS. Clinical Relevance Progressive cataracts of highly distinctive morphology are an important feature of HHCS. Evaluation for this type of cataract may be of diagnostic value in patients with unexplained hyperferritinemia. Hereditary hyperferritinemia-cataract syndrome can be a cause of cataracts in pediatric patients even in the absence of any positive family history.

Adolescent↗

Mapped serial excision for periocular lentigo maligna and lentigo maligna melanoma.

PURPOSE: To report the early cure rate for periocular lentigo maligna (LM) and LM melanoma (LMM), using modified Mohs surgery with vertically cut paraffin-embedded sections (mapped serial excision [MSE]). A secondary aim was to identify differences in the clinical features and outcomes between periocular LM and LMM and those found elsewhere on the head and neck. DESIGN: Prospective, noncomparative, interventional case series. PARTICIPANTS: One hundred thirty-five patients undergoing 141 MSE procedures. METHODS: A prospective series of 141 MSE procedures for LM and LMM over a 10-year period (1993-2002) in a single-center Mohs surgical unit. MAIN OUTCOME MEASURES: Recurrence, site, size of LM or LMM, invasiveness, prior recurrence, clear margin of excision, size of final defect, and number of levels required for complete excision. RESULTS: One hundred forty-one MSE procedures, of which 23% (32/141) were for LMM and 19% (27/141) were for periocular lesions. Location or prior recurrence were not predictive of invasive disease; however, the size distribution of the initial lesion (P = 0.0354) and the final defect after MSE (P = 0.0183) were larger in LMM. Thirty-one percent of LM and 14% of LMM less than 1 mm thick required larger than 5-mm and 1-cm margins, respectively, for complete excision. Mean follow-up of 32 months (range, 1-100 months) revealed 4 recurrences (3%), of which two were periocular (P = 0.188). CONCLUSIONS: Our review is the largest prospective series of MSE for LM and LMM and suggests that it is the treatment of choice in these forms of melanoma. Mapped serial excision offers a high early cure rate in conjunction with tissue conservation, which is of particular relevance in the periocular region. There were no significant differences between periocular LM and LMM and those found elsewhere in the head and neck region. It also appears that the current recommendations of 5-mm margins for in situ melanoma (LM) and 1-cm margins for melanoma less than 1 mm thick are insufficient for complete excision of LM or LMM, emphasizing the importance of margin-controlled excision of these lesions.

Adult↗

Craniopharyngioma: a review of long-term visual outcome.

PURPOSE: To assess the clinical presentation and long-term visual outcome in a series of patients with craniopharyn-gioma. METHODS: Retrospective case review. RESULTS: Thirty-six patients were reviewed, comprising 19 female patients and 17 male patients. The age range was 2-77 years with a bimodal distribution of 17 children (mean age 10 years) and 19 adults (mean age 47 years). Blurred vision was the most common visual complaint (23 cases, 64%) and headache the most frequent systemic complaint (19 cases, 53%). The average duration of systemic symptoms was 45 weeks compared to 10 weeks for visual symptoms. Deficits in visual acuity occurred in 13 patients (36%) and showed no significant change from initial presentation to final review. Sixteen patients (44%) had bitemporal hemianopia on presentation and pleomorphism (change from one type of visual field defect to another) occurred in 11 patients. Recurrence of tumour occurred in 15 patients (42%) and was more likely in children (59%) than adults (26%). The mean time period to recurrence was 7 years. The average follow-up period for all cases was 10 years. CONCLUSION: Patients with craniopharyngioma generally present late, and the visual symptoms are often preceded by a long history of systemic symptoms. Children are more likely to present with systemic symptoms than adults. Visual field pleomorphism is a feature of craniopharyngioma and occurred in one-third of the patients. Local recurrence is common. Although magnetic resonance imaging is the recommended means of follow up, regular neuro-ophthalmic review is useful in the early detection of anterior visual pathway compression by recurrent tumour.

Adolescent↗

Frontoethmoidal osteoma: a stereotactic-assisted sino-orbital approach.

A 28-year-old woman had an extensive osteoma arising in the frontoethmoidal sinuses and involving the orbital roof. The tumor was successfully removed by means of a combined sinus endoscopic and orbital approach, with the assistance of stereotactic localization. The patient had postoperative ptosis, diplopia, and supraorbital nerve anesthesia, which all resolved over a 3-month period. A stereotactic-assisted, sino-orbital approach to selected frontoethmoidal osteomas may provide a viable alternative to an orbitocranial approach.

Adult↗